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Human HARS Recombinant Protein (RPPB1797)

SKU:
RPPB1797
Product type:
Recombinant Protein
Size:
10ug
Species:
Human
Target:
HARS
Synonyms:
Histidyl-tRNA synthetase cytoplasmic
Synonyms:
Histidine--tRNA ligase
Synonyms:
HisRS
Synonyms:
HARS
Source:
Escherichia Coli
Uniprot:
P12081
€245
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Description

Product Name:Human HARS Recombinant Protein
Product Code:RPPB1797
Size:10µg
Species:Human
Target:HARS
Synonyms:Histidyl-tRNA synthetase cytoplasmic, Histidine--tRNA ligase, HisRS, HARS, HRS, FLJ20491.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The HARS solution (1mg/ml) contains 20mM Tris-HCl buffer(pH 8.0), 10% glycerol, 1mM DTT and 0.1M NaCl.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks.Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 95.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSMAERAAL EELVKLQGER VRGLKQQKAS AELIEEEVAK LLKLKAQLGP DESKQKFVLK TPKGTRDYSP RQMAVREKVF DVIIRCFKRH GAEVIDTPVF ELKETLMGKY GEDSKLIYDL KDQGGELLSL RYDLTVPFAR YLAMNKLTNI KRYHIAKVYR RDNPAMTRGR YREFYQCDFD IAGNFDPMIP DAECLKIMCE ILSSLQIGDF LVKVNDRRIL DGMFAICGVS DSKFRTICSS VDKLDKVSWE EVKNEMVGEK GLAPEVADRI GDYVQQHGGV SLVEQLLQDP KLSQNKQALE GLGDLKLLFE YLTLFGIDDK ISFDLSLARG LDYYTGVIYE AVLLQTPAQA GEEPLGVGSV AAGGRYDGLV GMFDPKGRKV PCVGLSIGVE RIFSIVEQRL EALEEKIRTT ETQVLVASAQ KKLLEERLKL VSELWDAGIK AELLYKKNPK LLNQLQYCEE AGIPLVAIIG EQELKDGVIK LRSVTSREEV DVRREDLVEE IKRRTGQPLC IC

Histidyl-tRNA synthetase (HARS) functions to catalyze the aminoacylation of tRNAs by their corresponding amino acids. HARS is a member of the class II family of aminoacyl-tRNA synthetases. HARS is responsible for the synthesis of histidyl-transfer RNA, which is vital for the incorporation of histidine into proteins. HARS is a frequent target of autoantibodies in the human autoimmune disease polymyositis/dermatomyositis.

HARS Human Recombinant fused with a 23 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 532 amino acids (1-509 a.a.) and having a molecular mass of 59.4kDa. The HARS is purified by proprietary chromatographic techniques.

UniProt Protein Function:HARS: Defects in HARS are a cause of Usher syndrome type 3B (USH3B). USH3B is a syndrome characterized by progressive vision and hearing loss during early childhood. Some patients have the so-called 'Charles Bonnet syndrome,' involving decreased visual acuity and vivid visual hallucinations. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa with sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH3 is characterized by postlingual, progressive hearing loss, variable vestibular dysfunction, and onset of retinitis pigmentosa symptoms, including nyctalopia, constriction of the visual fields, and loss of central visual acuity, usually by the second decade of life. Belongs to the class-II aminoacyl-tRNA synthetase family.
UniProt Protein Details:

Protein type:EC 6.1.1.21; Translation; Ligase

Chromosomal Location of Human Ortholog: 5q31.3

Cellular Component: cytoplasm; cytosol; mitochondrion

Molecular Function:histidine-tRNA ligase activity

Biological Process: histidyl-tRNA aminoacylation; mitochondrial translation; tRNA aminoacylation for protein translation

Disease: Charcot-marie-tooth Disease, Axonal, Type 2w; Usher Syndrome, Type Iiib

NCBI Summary:Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. The protein encoded by this gene is a cytoplasmic enzyme which belongs to the class II family of aminoacyl-tRNA synthetases. The enzyme is responsible for the synthesis of histidyl-transfer RNA, which is essential for the incorporation of histidine into proteins. The gene is located in a head-to-head orientation with HARSL on chromosome five, where the homologous genes share a bidirectional promoter. The gene product is a frequent target of autoantibodies in the human autoimmune disease polymyositis/dermatomyositis. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2012]
UniProt Code:P12081
NCBI GenInfo Identifier:135123
NCBI Gene ID:3035
NCBI Accession:P12081.2
UniProt Secondary Accession:P12081,B4DHQ1, B4DY73, D6REN6, J3KNE5,
UniProt Related Accession:P12081
Molecular Weight:54,847 Da
NCBI Full Name:Histidine--tRNA ligase, cytoplasmic
NCBI Synonym Full Names:histidyl-tRNA synthetase
NCBI Official Symbol:HARS  
NCBI Official Synonym Symbols:HRS; CMT2W; USH3B  
NCBI Protein Information:histidine--tRNA ligase, cytoplasmic
UniProt Protein Name:Histidine--tRNA ligase, cytoplasmic
UniProt Synonym Protein Names:Histidyl-tRNA synthetase; HisRS
Protein Family:Histidine--tRNA ligase
UniProt Gene Name:HARS  
UniProt Entry Name:SYHC_HUMAN

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